Scleroderma

Definition

Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are three major forms of the disease:

Localized scleroderma (can be morphea or linea)—Usually affects only the skin in isolated parts of the body. This form is less serious.

Systemic scleroderma—Affects widespread areas of skin and/or internal organs, most often the lungs. Certain categories of this form of scleroderma are more serious and can be fatal.

Overlap syndrome—May involve features of scleroderma and features of other autoimmune syndromes.

Causes

Overproduction of collagen and other connective tissue proteins are the main features of scleroderma. It is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production.

Risk

Scleroderma is more common in women. The morphea type of scleroderma usually strikes people around 20-40 years old. Linear scleroderma often occurs in children. Systemic scleroderma is more likely to occur in people aged 30-50 years old.

Other factors that may increase your chance of developing scleroderma include:

Family members with autoimmune disorders

Occupational chemical exposure, such as silica, ketones, or welding fumes

Symptoms

Localized Scleroderma

Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:

Hard patches on the skin, most often on the face or trunk (morphea)

Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)

Systemic Scleroderma

This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with Raynaud's phenomenon. This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.

Kidney Problems and/or High Blood Pressure

Raynaud's Phenomenon

Restricted Mobility

Physical therapy and exercise are important to maintain circulation, joint flexibility, and muscle strength.

Prevention

There are no current guidelines to prevent scleroderma.

Content was created using EBSCO’s Health Library. Edits to original content made by Rector and Visitors of the University of Virginia. This information is not a substitute for professional medical advice.